Pathology

Question 1

colour of type 1 skeletal muscle fibres

Answer 1

red

Question 2

colour of type 2 skeletal muscle fibres

Answer 2

white

Question 3

features of type 1 skeletal muscle fibres

Answer 3

large mitochondria and high myoglobin

resistant to fatigue due to ability to regenerate ATP

Question 4

features of type 2 skeletal muscle fibres

Answer 4

small mitochondria and large motor end plates

fatigue rapidly, good for short bursts of energy

Question 5

CK levels in dystrophies

Answer 5

high

200-300x normal

Question 6

CK levels in inflammatory myopathies

Answer 6

intermediate 20-30x normal

Question 7

CK levels in neurogenic disorders

Answer 7

low 2-5x normal

Question 8

features of DMD

Answer 8

proximal weakness

pseudoypertrophy of the calves

Question 9

Mutation in DMD

Answer 9

dystrophin gene on long arm X chromosome

Question 10

Effect of mutation in DMD

Answer 10

alterations in anchorage of actin cytoskeleton to basement membrane
uncontrolled calcium entry into cells

Question 11

Histology in DMD

Answer 11

muscle fibre necrosis and phagocytosis

chronic inflammation and fibrosis

Question 12

Which is worse - DMD or Becker MD?

Answer 12

DMD

Question 13

Where is the mutation in BMD

Answer 13

dystrophin gene

Question 14

Most common type of muscular dystrophy

Answer 14

myotonic dystrophy

Question 15

Inheritance of myotonic dystrophy

Answer 15

autosomal dominant

Ch19/Ch3

Question 16

Histology of myotonic dystrophy

Answer 16

atrophy of type 1 fibres 
central nuclei 
ring fibres 
fibre necrosis 
fibrofatty replacement

Question 17

Pathophysiology of polymyositis

Answer 17

cell-mediated immune response to muscle antigens

Question 18

Histology of polymyositis

Answer 18

endomysial lymphocytic infiltrate, invasion of muscle by CD8+ T lymphocytes

Question 19

What is dermatomyositis?

Answer 19

skin changes + polymyositis

Question 20

What is the association of malignancy with dermatomyositis

Answer 20

10%

Question 21

Histology of dermatomyositis

Answer 21

B lymphocytes and CD4+ T cells

Question 22

What is motor neurone disease

Answer 22

progressive degeneration of the anterior horn cells

characterised by denervation atrophy, fasciculation and weakness

Question 23

What is spinal muscular atrophy

Answer 23

degeneration of the anterior horn cells in the spinal cord

Question 24

How is spinal muscular atrophy inherited?

Answer 24

autosomal recessive

Ch5

Question 25

What kind of disease is myasthenia gravis

Answer 25

autoimmune

Question 26

What are the characteristics of myasthenia gravis

Answer 26

weakness proptosis fatigue dysphagia`

Question 27

what are the antibodies in myasthenia gravis

Answer 27

anti-nicotinic ACh receptor antibodies

Question 28

what do 25% of myasthenia gravis patients also have?

Answer 28

thymoma

Question 29

what is rhabdomyolysis?

Answer 29

breakdown of skeletal muscle

Question 30

what is polyarteritis nodosa

Answer 30

inflammation and fibrinoid necrosis of small/medium arteries

Question 31

how is polyarteritis nodosa diagnosed

Answer 31

biopsy shows fibrinoid necrosis of vessels | serum pANCA

Question 32

What does polymyalgia rheumatica respond well to

Answer 32

corticosteroids

Question 33

what is temporal arteritis also known as

Answer 33

giant cell arteritis

Question 34

what are patients with GCA at risk of

Answer 34

blindness

Question 35

Complaint in GCA

Answer 35

headache and scalp tenderness

Question 36

diagnosis of GCA

Answer 36

temporal artery biopsy | ESR is raised

Question 37

what is scleroderma?

Answer 37

excessive fibrosis of organs and tissues due to excessive collagen production

Question 38

CREST syndrome

Answer 38

``` calcinosiss raynauds esophageal dysfunction sclerodactyly telangiectasia ```